Schwannomas are benign tumors, usually solitary, encapsulated, slow-growing, which have their origin in differentiated neoplastic Schwann cells with extramedullary intradural usual development related to nerve roots. The melanotic schwannoma is a variant of these tumors whose location in almost one third of cases is on the posterior spinal nerve root, with a nonspecific clinical presentation. Magnetic resonance imaging is the most widely used test for the diagnosis, revealing hyperintense T1-weighted sequences and hypointense T2-weighted sequences. Diagnostic confirmation is obtained by histological and immunohistochemical studies, in which there is intense cytoplasmatic pigmentation. There are two distinct types of melanotic schwannomas: sporadic and psammomatous, the latter related to the called Carney complex, a form of multiple endocrine neoplasm with familiar character. In literature we found few cases of these neoplasms, the largest series consisting of five cases. The objective of this study is to report a rare case of melanotic schwannoma of the lumbar spine of the sporadic type of extramedullary location. We also present a brief review of the literature containing the main characteristics of the tumor, including its different forms, differential diagnoses, data from histological and immunohistochemical studies as well as the currently recommended approach in order to contribute to a better understanding of this neoplasm.

CONTEXT AND OBJECTIVE: Hereditary angioedema (HAE) with C1 inhibitor deficiency manifests as recurrent episodes of edema involving the skin, upper respiratory tract and gastrointestinal tract. It can be lethal due to asphyxia. The aim here was to evaluate the response to therapy for these attacks using icatibant, an inhibitor of the bradykinin receptor, which was recently introduced into Brazil. DESIGN AND SETTING: Prospective experimental single-cohort study on the efficacy and safety of icatibant for HAE patients. METHODS: Patients with a confirmed HAE diagnosis were enrolled according to symptoms and regardless of the time since onset of the attack. Icatibant was administered in accordance with the protocol that has been approved in Brazil. Symptom severity was assessed continuously and adverse events were monitored. RESULTS: 24 attacks in 20 HAE patients were treated (female/male 19:1; 19-55 years; median 29 years of age). The symptoms were: subcutaneous edema (22/24); abdominal pain (15/24) and upper airway obstruction (10/24). The time taken until onset of relief was: 5-10 minutes (5/24; 20.8%); 10-20 (5/24; 20.8%); 20-30 (8/24; 33.4%); 30-60 (5/24; 20.8%); and 2 hours (1/24; 4.3%). The time taken for complete resolution of symptoms ranged from 4.3 to 33.4 hours. Adverse effects were only reported at injection sites. Mild to moderate erythema and/or feelings of burning were reported by 15/24 patients, itching by 3 and no adverse effects in 6. CONCLUSION: HAE type I patients who received icatibant responded promptly; most achieved improved symptom severity within 30 minutes. Local adverse events occurred in 75% of the patients.

Objective: The prevalence of osteoporosis in patients with Adolescent Idiopathic Scoliosis (AIS) is believed to be higher than in the general adolescent population. An alternative to radiology for the characterization of bone mineral density may be through correlative indexes like the Osteorisk index, which is easy to access and low in cost, and which helps the doctor in the request for Bone Densitometry. Our belief that osteoporosis can affect the evolution and treatment of AIS was what motivated us to conduct this study. Our objective was to subjectively evaluate bone mineral density by the Osteorisk index in patients with AIS. Methods: Healthy patients (control group, n=30) and patients with AIS (n = 30) were evaluated, documenting age, weight and height, and establishing the Osteorisk. The unpaired Student t test was performed, with a level of significance of p <0.05. Results: The mean Osteorisk found for the patients with AIS was 6.38 ± 2.2 while in the control group, it was 8.27 ± 2.14, which represents a low risk of developing osteoporosis in both groups. Comparing these means between the groups, a lower Osteorisk was observed in the AIS group. Conclusion: Our study showed that there is low risk of developing osteoporosis in patients with AIS. Level of Evidence I, Prospective study.

To carry out the cross-cultural adaptation of Eular Sjgrens Syndrome Patient Reported Index (ESSPRI) for Portuguese language and evaluate its psychometric properties.Cross-sectional study of patients with primary Sjgrens syndrome (SS). The psychometric properties (intraobserver reproducibility and construct validity) were studied. In construct validity, ESSPRI was compared with the Patients Global Assessment (PGA), Profile of Fatigue and Discomfort (Profad), Sicca Symptoms Inventory (SSI) and Functional Assessment of Chronic Illness Therapy (Facit-F). Statistical tests used were:Cronbachs alpha, intraclass correlation coefficient (ICC), Bland-Altman method and Spearman coefficient. A value of p 0.05 was considered significant.There was no difference between versions in both languages; thus, a Brazilian consensual version was obtained. All subjects were women aged 49.4 11.6 years, with onset of symptoms of 7.2 5.4 years, and time of diagnosis of 3.0 3.3 years. The mean ESSPRI was 6.87 1.97. The intraobserver reproducibility was high and significant (0.911) and, with Bland-Altman method, there was no systematic bias in the agreement of measures among evaluations. A moderate correlation of ESSPRI with all tested instruments was observed.The Brazilian Portuguese version of ESSPRI is a valid and reproducible version.